Nonfollicular papules in the bilateral inframammary region

BACKGROUND A 56-year-old female with a history of diabetes, hypertension, and hyperlipidemia presents with a 6 month history of rash under bilateral breasts for which treatment with nystatin powder and ketoconazole cream demonstrated no improvement. On examination, loose atrophic skin andwhite-to-yellow nonfollicular papules coalescing into plaques in the bilateral inframammary region with incomplete involvement of the folds were observed (Fig 1). A punch biopsy of the area showed degeneration and fragmentation of elastic fibers (Fig 2). Verhoeff e Van Gieson (VVG) staining confirmed the near absence of elastic fibers within the papillary dermis (Fig 3).


BACKGROUND
A 56-year-old female with a history of diabetes, hypertension, and hyperlipidemia presents with a 6 month history of rash under bilateral breasts for which treatment with nystatin powder and ketoconazole cream demonstrated no improvement.On examination, loose atrophic skin and white-to-yellow nonfollicular papules coalescing into plaques in the bilateral inframammary region with incomplete involvement of the folds were observed (Fig 1 ).A punch biopsy of the area showed degeneration and fragmentation of elastic fibers (Fig 2).Verhoeff e Van Gieson (VVG) staining confirmed the near absence of elastic fibers within the papillary dermis (Fig 3).  A. Pseudoxanthoma elasticum e Incorrect.Papillary dermal elastosis (PXE) resembles PXE-PDE, but onsets at a younger age with ocular and cardiovascular manifestations. 1 On histology with the von Kossa stain, PXE illustrates fragmentation and calcification of elastic fibers. 1 B. Pseudoxanthoma-like papillary dermal elastolysis e Correct.This is a rare, benign elastic tissue disorder, mainly in women with a clinical similarity to PXE without systemic manifestations. 1 It presents as asymptomatic white-to-yellow nonfollicular papules forming plaques commonly located in sunexposed areas like the neck. 1 Verhoeff e Van Gieson or orcein stains illustrate a total or partial loss of elastic fibers in the papillary dermis in a linear band formation, without calcification, unlike PXE. 2 C. Papillary dermal elastosis e Incorrect.This appears like PXE-PDE with asymptomatic papules on the neck and upper back, but histology of PDE demonstrates foci of clumped elastic fibers in the papillary dermis with normal reticular dermis. 2 D. White fibrous papulosis e Incorrect.WFP is similar to PXE-PDE but distinguished from it due to equal presence in both sexes with nonconfluent paler papules, usually on the nape of the neck, lacking the cobblestone appearance. 2 Histology shows elimination of the papillary dermal elastic plexus with the upper reticular dermis illustrating fibrosis not seen in PXE-PDE. 2 E. Mid-dermal elastolysis e Incorrect.This is another rare elastolytic disorder presenting with 3 distinct variations: well-demarcated patches of wrinkles on the trunk and proximal extremities (type I); follicular papules on the lateral neck (type II); and reticular erythema with wrinkling. 2Histology illustrates paucity of elastic fibers in the mid-dermis, with occasional lymphohistiocytic infiltrate and elastophagocytosis. 2 A. UV damage e Incorrect.UV exposure is one of the proposed etiologic theories of PXE-PDE.However, UV exposure as the main cause of PXE-PDE is debated as involvement of the axillae, and inframammary folds as in our patient, can occur.In patients with increased melanophages in the dermis, a marker of UV damage in chronic heliodermatitis, the UV radiation etiologic theory may play a role in PXE-PDE development. 2 Genetics e Incorrect.Although less likely, a genetic cause of PXE-PDE is supported by one report of a familial case of PXE-PDE. 3 C. Aging process e Incorrect.The thinning of oxytalan and elastin within the papillary dermis associated with intrinsic aging has also been postulated as a cause of PXE-PDE and why it occurs in middle-aged individuals.2 However, this does not explain why PXE-PDE occurs largely in women and not both sexes.D. Abnormal elastin synthesis e Incorrect.The dysfunctional elastin synthesis theory has the most supportive evidence with studies using immunohistochemical staining revealing immature elastic fibers in the mid-dermis with a defect in elastin rather than fibrillin-1. 2is is further supported by case reports of PXE-PDE in patients on high-dose or prolonged steroid treatment for rheumatologic conditions, illustrating the effect of glucocorticoids on elastin gene expression and messenger ribonucleic acid. 2 E. Environmental exposure e Correct.Although UV damage is a proposed theory for the development of PXE-PDE, other environmental exposures have not been elucidated as contributing to this disease process.

B.
Fraxel laser e Incorrect.Fraxel is a nonablative fractional resurfacing laser that has shown up to a 50% improved appearance of the skin after 3 sessions in 1 case report. 4 Clear and brilliant laser e Incorrect.This is also a nonablative fractional resurfacing laser that may be used to treat the cosmetic concerns of PXE-PDE.

D.
Oral retinoids e Correct.To date, oral retinoids have not been utilized in the literature to treat PXE-PDE.Additionally, because PXE-PDE is a benign condition, the risks of adverse effects from oral retinoids outweigh the benefits of any potential improvement.

E.
No treatment e Incorrect.Once PXE is ruled out, the disease remains benign, and treatment is not necessary unless cosmetic concern exists.

Question 2 :
Which of the following is NOT a proposed etiologic theory for the development of PXE-PDE? A. Ultraviolet (UV) radiation B. Genetics C. Aging process D. Abnormal elastin synthesis E. Environmental exposure Answers:

Question 3 :
Which of the following is NOT a treatment option? A. Topical retinoids B. Fraxel laser C. Clear and brilliant laser D. Oral retinoids E. No treatment Answers: A. Topical retinoids e Incorrect.Most treatments for PXE-PDE are ineffective, however there have been case reports in which topical retinoids have had minimal to marked change with desirable results.